Showing papers on "Amyotrophic lateral sclerosis published in 1977"

Preservation of a certain motoneurone group of the sacral cord in amyotrophic lateral sclerosis: its clinical significance.

Abstract: Histopathological description of a special nerve cell group of the sacral spinal cord in five cases of amyotrophic lateral sclerosis is presented. Remarkable preservation of this group of neurones (the X group of Onuf) was noted in each case, and this is discussed with particular reference to vesicorectal function, since it is well known that the function of bladder and rectal sphincters is often intact until the latest stage of the illness.

Psychological characteristics of patients with amyotrophic lateral sclerosis (ALS).

Abstract: Previous research has suggested that patients with amyotrophic lateral sclerosis (ALS) demonstrate similar psychological characteristics that distinguish them from the medically ill populations, and that these findings may bear etiologic and prognostic importance. In this report, a larger sample was studied and the prior findings were not replicated. Specifically, ALS patients were not found to have a more internal locus of control, or to use denial as a defense more than a group of patients with inoperable neoplastic disease. Although dysphoria was frequently found in ALS patients, only 22% demonstrated clinical depressive syndromes.

Calcium metabolism in amyotrophic lateral sclerosis.

Abstract: Calcium metabolism was studied prospectively in 12 patients with amyotrophic lateral sclerosis. Two patients showed mild hypocalcemia, malabsorption of calcium, and elevated plasma parathyroid hormone concentrations. Serum 25-hydroxyvitamin D was decreased in one and low-normal in the second. These two patients and a third showed aminoaciduria on thin layer chromatography. Calcium metabolism was apparently restored to normal by dihydrotachysterol, a vitamin D analog, but no improvement in neurologic function resulted. Bone radiographs taken in search of metabolic bone disease showed a significant increase in the incidence of congenital vertebral anomalies in the ALS patients (50% versus 8%). The relationship of the abnormalities in calcium metabolism and in vertebral structure to the etiology of motor neuron disease is not known.

Amyotrophic lateral sclerosis in dizygotic twins

Abstract: A pair of dizygotic twins developed typical amyotrophic lateral sclerosis during adulthood. The concordance for this disease in these two patients of nonconsanguineous parentage with no family history of the disorder suggests the possibility of sublethal intrauterine injury to anterior horn cells. Infectious or toxic exposure during the twins9 intimately shared milieu in the prenatal period could have resulted in a neuronal “abiotrophy” that would not have become clinically apparent until decades later. If such a prenatal neuronal injury plays a role in the pathogenesis of amyotrophic lateral sclerosis, it becomes clear that attempts at experimental animal transmission of the disease from affected adults would fail even if the offending agent were viral.

Prognosis in Hereditary Amyotrophic Lateral Sclerosis

Abstract: • Two different forms of hereditary amyotrophic lateral sclerosis (ALS) has been separated according to duration of illness. A rapid course with short survival as seen in sporadic ALS is usual, but a comparatively benign type with a mean survival of 12 years has been reported in some families. Four patients from an ALSafflicted family with five affected members in three generations were examined and then followed up. A conspicuous variability in progression among the patients was observed, with death occurring from 26 months to 12 years after onset; one patient is alive 13 years after onset. Wide differences were also found with respect to initial site of involvement and pyramidal tract signs. Three other families with this mixed pattern of prognosis have been reported previously. Affected individuals within involved families had either short or long duration of the disease, rather than displaying a continuum. However, in view of the existence of a type of hereditary ALS with marked intrafamilial variability, prognosis, even in the presence of previous benign cases, should be cautiously given.

Amyotrophic Lateral Sclerosis: Recent Research Trends

Abstract: This book reports current research that may be important for understanding the etiology and therapy of amyotrophic lateral sclerosis (ALS). It is not meant as a review of what is known about ALS, and the reader will find very little to aid his understanding of the clinical disorder. The book focuses on the anterior horn cell of the spinal cord. The strength of the book is the excellent description of their own researches by investigators engaged in understanding the physiology and pathology of these cells. This, combined with an excellent bibliography, will be of great help to investigators who are studying or planning studies of the basic pathophysiology of amyotrophic lateral sclerosis. The initial chapters include an excellent review of the structural substrate of protein synthesis in the neurons as well as a discussion of the axonal transport systems. Motor neuron physiology and chemistry are discussed as they may possibly

Current status of the search for virus in amyotrophic lateral sclerosis (ALS).

Abstract: Clinicians have noted resemblance between ALS with predominantly lower motor involvement and a chronic type of poliomyelitis. Occasional cases of ALS show lymphocytic infiltrations in the neuropathology. Cytoplasmic inclusion bodies as well as possible transmission of ALS to the monkey, were noted by Soviet investigators. Major questions about the latter remain, but inclusion bodies have been seen in cases outside the USSR, and search for a virus has been pursued. So far, efforts at culture, including the technique of co-cultivation, have not been successful in 3 different laboratories using material from ALS autopsied within 2 hours of death. Nor has such material and specimens from other cases produced transmissible disease in animals of laboratories where kuru, Jakob's disease and SSPE have been transmitted. Study of serum antibodies has also been negative. These negative culture, transmission and antibody studies do not exclude a viral etiology: in kuru, for example, many years passed before transmission was demonstrated, which remains the only evidence of kuru's infectivity. Evidence of an immunologic disorder in ALS has been obtained from kidney biopsies, the lack of any clinical benefit from immunosuppression may indicate that the kidney finding reveals a response to infection, though whether viral or even relevant is unknown. Two recent ALS cases will be illustrated in which virus-like particles were demonstrated post mortem by electronmicroscopy. In one, myxovirus-like tubules were seen mainly in motor neurons; in the other, picorna-virus-like crystals were found in skeletal muscles. Different viruses can cause identical disease, but 2 different viruses in different sites in ALS also suggest that at least one type of particle is an adventitious finding of no general significance. An additional impetus to continue the search is the slowly progressive type C-virus myelitis in wild mice. This disease has many dissimilarities to ALS, including different morphology of the virus, but may provide a model for slow-virus amyotrophy.

Dominantly Inherited Amyotrophic Lateral Sclerosis (Motor Neuron Disease)

Abstract: This is paper #46 from the Department of Human Genetics of the Medical College of Virginia and was supported in part by Grant #C-187 from the National Foundation-March of Dimes. Correspondence and reprint requests to Lpuise E. Wilki!)s, Box 33, Medical College of Virginia, Richmond, Virginia 23298. sensory impairment.1 Later, th~ syndromes of progressive bulbar palsy (PBP) and progressive muscular atrophy (PMA) were recognized to be variations of the same pathological process, and ALS was used as an inclusive term to refer to these syndromes as well. Although some authors reserve the term ALS for the specific syndrome of mixed upper. and lower motor neuron lesions and use the term "motor neuron disease" to refer to the constellation of syndromes, most

Characteristics of Dysarthric Speech due to Cerebellar disorders, Pseudobulbar Palsy and Amyotrophic Lateral Sclerosis

Abstract: With the objective of analyzing and describing the characteristic patterns of deviant speech of patients in three discrete neurologic groups(cerebellar degeneration, pseudobulbar palsy and amyotrophic lateral sclerosis), speech samples of each patient consisting of sentences and repetitions of syllables were used for rating on each of 39 predetermined dimensions of speech and voice. Analysis based on the means of the ratings of the eight judges on each patient on each dimension yields results leading to the following conclusions: (1) Ratings for sentence samples were higher than those for repetitions of syllables. (2) Some of the dimensions highly deviant in each group were common to all three groups, such as imprecision of consonants and slow rate, while others were characteristic of each group. (3) Speech and voice of the group with Cerebellar disorders were found to be characterized by irregular articulatory and phonatory break down, whereas those of the other two groups were found to be lacking in resonatory-articulatory and phonatory competence, the two groups varying in degree in some of the dimensions. (4) The above characteristics seem to mirror the general abnormalities of motor functions in each group. (5) Our findings are comparable to those of Darley et al. and Hirose with some exceptions one of which is considered to be due to the difference of language.